Care of the pregnant patient with
Sickle Cell Disease (GL914)
Approval
Approval Group
Job Title, Chair of Committee
Maternity & Children’s Services
Clinical Governance Committee
Date
th
Chair, Maternity Clinical
Governance Committee
13 November
2015
Change History
Version
Date
Author, job title
Reason
1.0
Trust requirement
2.0
Reviewed
3.0
March
2010
Lakshmi Shanmugasundaram
(Consultant Obstetrician),
Sarah Banks (Nurse
practitioner in
haemoglobinopathy)
Reviewed
4.0
May 2013
Brian Reid (Consultant
Obstetrician), Sarah Banks
(Nurse practitioner in
haemoglobinopathy), Maged
Shendy (Speciality doctor)
Reviewed
5.0
Aug 2015
Miss J Siddall (Consultant
Obstetrician)
Reviewed
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician,
Group Director Urgent Care
Date:
Review Date:
Version:
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
November 2015
November 2017
th
V5.0 ratified 13 Nov 2015
Mat CG mtg
Page 1of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)
1.0 Booking and antenatal care:

1.1
A management plan should be made in conjunction with the patient,
obstetrician, consultant haematologist and the obstetric anaesthetist should also
be informed.
Discussion of pregnancy and associated risks. Discuss maternal and
fetal risks of pregnancy
Maternal risks
 Painful crisis Infection - affects > 50%
pregnancies with SCD
 Chest syndrome
 Hypertension & pre Eclampsis
 Worsening anaemia
 Increased risk of cholecystitis
 Increased cesarean rate
 Thrombosis
1.2
Fetal/neonatal risks
 Miscarriage
 Increased perinatal mortality
 Intrauterine growth retardation and low
birth weight
 Premature delivery
Confirmation of diagnosis
o A full history should be taken, to include
 Frequency and management of any crisis
 Acute and chronic complications
 Transfusion history
 Infections
o The woman should have a clinical examination and baseline oxygen
saturations and blood pressure should be taken. Further investigations
will depend on clinical findings.
o Maternal bloods should be sent for the following as part of their obstetric
booking investigations , HbS%, red cell folate, ferritin, urea and
electrolyte (U&Es), liver function tests (LFTs) , cytomegalovirus (CMV)
status, Hepatitis C and HIV ( if not already done), and a group and save.
o An echocardiogram and retinal screen should be performed if it has not
been done already
o Folic acid 5 mg daily, continued throughout pregnancy.
o Prophylactic antibiotics should continue if they are already taking them. If
not, an individual decision whether to commence antibiotics can be
discussed with the woman.
o Routine blood transfusions are not usually necessary for an
uncomplicated pregnancy but should be considered on an individual
basis for each woman if they have a poor obstetric or medical history.
This must be agreed to by a haematologist. If the woman has signs of
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 2 of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)
transfusional iron overload, a cardiac assessment is required to exclude
cardiomyopathy.
o The woman should be advised to seek prompt medical attention at the
earliest sign of infection or complications relating to their SCD.
o Ultrasound to assess viability and confirm gestation
1.3
Prenatal diagnosis
o Arrangements should be made for the woman’s partner to be screened
for a haemoglobinopathy. This can be done via the antenatal screening
coordinator, ext.7292. If the couple are found to be ‘at risk’ of having a
child with a major haemoglobinopathy the couple should be counselled
together when possible with the antenatal screening coordinator
(ext.7292) and the haematology nurse specialist (bleep 331).
o Depending on the outcome of parental investigation, invasive prenatal
diagnostic testing may be appropriate:

Both partners have SCD then all children will have SCD

One partner SCD + other trait – 1 in 2 trait, 1 in 2 SCD

One partner SCD + normal – all children will have trait

Both partners trait – 1 in 4 trait or SCD, 1 in 2 normal
o In such cases, Chorion Villus Sampling at 11-13 wks or Amniocentesis at
16 wks can be offered
2.0 Subsequent antenatal visits
The woman should be followed up every 4 wks unless clinically indicated, then
every 2 wks from 28 weeks gestation until 36 weeks, then weekly until term.
o FBC and haematocrit should be checked monthly, if iron deficiency is
suspected, confirm by iron studies before prescribing iron medication.
o Continue folic acid 5 mg.
o LFTs and renal function should be checked every 4/52
o Blood pressure should be checked every visit, baseline blood pressure
will be generally lower than in the non-sickle population and relatively
modest changes may be an early indication of pregnancy induced
hypertension.
o If red cell alloantibodies are detected, serology must be sent every 2/52
from 16 weeks gestation, or as directed by the National blood service.
o Midstream urinalysis should be performed routinely at each visit and
midstream urine (MSU) sent to microbiology where indicated. If the MSU
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 3 of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)
confirms significant bacterial growth, the woman should be treated with
broad spectrum antibiotics.
o Ultrasound scans should be performed:
a)
b)
c)
d)
20 weeks gestation
Consider uterine artery doppler at 24 weeks
Scan for biometry at 28, 32 and 36 weeks
If IUGR, scan more frequently and check UA Doppler
Low threshold for admission especially if limb, bone, abdominal, chest pain after
28 weeks. If admitted during pregnancy, use low molecular weight heparin for
thrombopropylaxis and compression stockings.
3.0 Management of complications
3.1
Infection
o Usually of urinary or respiratory origin, incidence of 50% of women with
SCD in pregnancy. The woman should have a full septic screen and be
treated with broad spectrum antibiotics to prevent a sickling crisis.
3.2
Painful Crisis
o Some women experience more painful crisis in pregnancy. If the crisis is
severe the woman will need admitting to hospital under joint care of the
obstetrician and haematologist.
o Ensure rest and warmth
o Give oxygen if hypoxic on monitoring of O2 saturation.
o Ensure adequate hydration – oral or intravenous fluids 3–4 liters. Strict
fluid balance essential.
o Pain relief




Paracetamol
NSAID’s should be used in caution in pregnancy and should not be
given in the first trimester or after 32 weeks
Pethidine is not recommended for the treatment of sickle pain.
Morphine , diamorphine or oxycodone are appropriate
Patient-controlled analgesia or subcutaneous pumps are occasionally
required.
o Investigations – FBC, reticulocytes, U&Es, group and screen, pulse
oximetry and arterial blood gases if appropriate.
o Microbiology – urine culture, blood cultures and throat swabs.
o Consider chest X-ray if chest involvement.
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 4 of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)
o Antibiotics are not routinely required unless evidence of infection, low
grade fever >38° C is common in painful crisis even in the absence of
infection.
o Low molecular weight heparin thromboprophylaxis and compression
stockings.
o Any transfusion should in liaison with hematologist.
o Fetal wellbeing should be assessed regularly with sonicaid /
cardiotocography as appropriate
3.3
Blood transfusion
o Although transfusion in pregnancy is well tolerated it carries a risk of
alloimmunisation, iron overload and transfusion transmitted infection.
o Indications for transfusion in pregnancy are:

*Hb <6g/dl

*Anaemia with cardio or respiratory compromise

*History of severe sickle related complications

*Patient already on a chronic transfusion programmes

*Twin pregnancies
o Routine guidelines for use of blood products should be maintained
including using full Rhesus compatible, Kell negative, CMV negative (in
non-immune individuals) red cells.
o Patients who are already on iron chelation treatment should be advised to
discontinue once pregnancy is confirmed. Those with significant iron
overload should be assessed by an endocrinologist and a cardiologist.
o
3.4
Jaundice
o Jaundice in SCD is due to an unconjugated hyperbilirubinaemia as a
result of haemolysis and may be increased during painful crisis. Any
increase in jaundice from steady state should be investigated further.
There is no risk of maternal haemolytic jaundice to the fetus.
4.0 Labour and Delivery

A normal vaginal delivery should be the aim, unless other factors supervene.
Labour should be actively managed and attention paid to the instructions below.
Epidural anaesthesia can be employed.
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 5 of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)

Dehydration, hypoxia, acidosis, infection and cold can precipitate painful crisis;
therefore should be avoided, especially in labour when there is already
increased stress on the baby. Paediatricians must be informed if the mother has
a history of recent opiate use or dependence.

Placental insufficiency is common, leading to fetal compromise during labour. In
view of that, the following is recommended:
o *IV fluids 3L/24hours. If PET present, seek consultant advice about fluid
replacement.
o *Oxygen, keep oxygen saturations <95%
o *Continuous electronic fetal monitoring
o *Use standard labour analgesia (e.g. nitrous oxide, opiates or epidural)
o *Prophylactic antibiotics to cover labour and puerperium (Co-amoxiclav
625 mg 8hrly or, if allergic, Erythromycin)
4.1
Analgesia
o For women receiving anticoagulation it is safe to proceed with epidural if
>12hours since the last low weight molecular heparin injection and
clotting screen is normal. Advice from the consultant anaesthetist should
be requested for women on therapeutic dose anticoagulation.
4.2
Caesarean section
o For lower segment caesarean section (LSCS), a regional anaesthetic is
preferred to a general one. GA would need more intensive monitoring and
increases the risk of postpartum complications.
o
Following delivery, all women should be monitored closely on the labour
wards high dependency area, or equivalent, and 4 hourly observations
of vital signs and oxygen saturations on room air (even on oxygen) until
the woman is mobilising. If oxygen saturations drop <92% the
Haematologist must be contacted as chest syndrome can be a risk
factor.
o Women who have had LSCS will prophylactic anticoagulation for 6
weeks postpartum.
5.0 Post-Partum

The woman is at risk for the first few weeks after delivery. The following is
recommended.
o Maintain IV hydration for the first 24 hours after delivery
o Prescribe 5 days of broad spectrum antibiotics post delivery
o Encourage early mobilization so as to avoid the increased risk of
thromboembolism, and thromboprophylaxis with LMWH for 6 wks, if other
risk factors exist
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 6 of 7
Maternity Guidelines – Care of the pregnant patient with sickle cell disease (GL914)
6.0 On discharge
A follow-up appointment should be made with the Haematology team 3 wks after
delivery. The General Practitioner and Community Midwife should be informed of
the patients discharge and her diagnosis
7.0 References
7.1
Pavord S, Hunt B, The obstetric haematology manual, 2010, Cambridge
university press: 28-44
7.2
Modell B, Harris R, Lane B et al. Informed consent in genetic screening for
Thalassaemia during pregnancy: audit from a national confidential inquiry.
British Medical Journal 2000; 320: 337–341
7.3
Serjeant GR. Sickle Cell Disease. Oxford: Oxford University Press, 1992.
Author:
Job Title:
Policy Lead:
Miss J Siddall
Consultant Obstetrician
Group Director Urgent Care
Location:
Maternity CG Shared drive/ Medical conditions & complications/ GL914
This document is valid only on Last printed 24/11/2015 11:49:00
Date:
Review Date:
Version:
November 2015
November 2017
th
V5.0 ratified 13 Nov
2015 Mat CG mtg
Page 7 of 7